RELYVRIO (sodium phenylbutyrate and taurursodiol)

RELYVRIO (sodium phenylbutyrate and taurursodiol)

Self-Administration – oral

Indications for Prior Authorization:

• Amyotrophic Lateral Sclerosis (ALS) - Indicated for the treatment of amyotrophic lateral sclerosis (ALS) in adults.

Coverage Criteria:

For diagnosis of amyotrophic lateral sclerosis (ALS):

• Dose does not exceed 6 g sodium phenylbutyrate/2 g taurursodiol (2 packets) daily; AND

• Patient is 18 years of age or older; AND

• Diagnosis of ALS is supported by neurogenic changes in electromyography (EMG); AND

• Patient has had ALS symptoms for less than or equal to 18 months; AND

• Patient has a percent (%) forced vital capacity (%FVC) or slowed vital capacity (%SVC) greater than or equal to 60% at the start of treatment; AND

• Patient does not require permanent noninvasive ventilation or invasive ventilation; AND

• Patient has had an inadequate response or intolerable side effects to riluzole, or patient will continue to take riluzole; AND

• Prescribed by or in consultation with a neurologist with expertise in the diagnosis of ALS

Reauthorization Criteria:

For diagnosis of amyotrophic lateral sclerosis (ALS):

• Dose does not exceed 6 g sodium phenylbutyrate/2 g taurursodiol (2 packets) daily; AND

• Documentation of positive response to therapy (e.g., slowed disease progression from baseline)

Coverage Duration:

• Initial: 6 months

• Reauthorization: 1 year

Authorization is not covered for the following:

The use of this drug for indications not listed in this policy does not meet the coverage criteria established by the Western Health Advantage (WHA) Pharmacy and Therapeutics (P&T) Committee.

Additional Information:

• Dosing

  • One packet should be emptied into a cup containing 8 ounces of room-temperature water and stirred vigorously. It should be taken orally or administered via a feeding tube within 1 hour of preparation and before a snack or meal.

  • The reconstituted suspension may be stored for up to 1 hour at room temperature. Any unused reconstituted suspension should be discarded after 1 hour.

• Patients with enterohepatic circulation disorder, pancreatic disorders, or intestinal disorders may lead to a decreased absorption of either component of the drug, and consultation with a specialist should be considered.

• Relyvrio has a high sodium content (1 packet contains about 464 mg sodium). Sodium intake should be monitored in patients with sodium sensitivity.

Policy Updates:

• 06/01/2023 – New policy approved by P&T

References:

• Paganoni S, Macklin EA, Hendrix S, et al. Trial of Sodium Phenylbutyrate–Taurursodiol for Amyotrophic Lateral Sclerosis. New England Journal of Medicine. 2020;383(10):919-930. 

• Andersen PM, Abrahams S, Borasio GD, et al. EFNS guidelines on the Clinical Management of Amyotrophic Lateral Sclerosis (MALS) - revised report of an EFNS task force. European Journal of Neurology. 

• Paganoni S, Hendrix S, Dickson SP, et al. Long-term survival of participants in the CENTAUR trial of sodium phenylbutyrate-taurursodiol in amyotrophic lateral sclerosis. Muscle & Nerve. 2021;63(1):31-39. 

• ClinicalTrials.gov: https://clinicaltrials.gov/ct2/show/study/NCT03127514. Accessed May 26, 2022. 

• Galvez-Jimenez N, Goyal NA. Disease-modifying treatment of amyotrophic lateral sclerosis. UpToDate Web site. http://www.uptodate.com. Accessed May 26, 2022. 

• Elman LB, McCluskey, L. Diagnosis of amyotrophic lateral sclerosis and other forms of motor neuron disease. UpToDate Web site. http://www.uptodate.com. Accessed May 26, 2022. 

• Relyvrio Prescribing Information. Amylyx Pharmaceuticals, Inc. Cambridge, MA. September 2022.